| Catalog No. | Size | Price (USD) |
| 10449-RP02-B-100 | 100 µg | $370.28 |
General Information
| Product name | Anti-ARSA Antibody (Biotin), Rabbit Polyclonal |
| Validated applications | ELISA (FAQ Protocol) |
| Species reactivity | Reacts with: Human |
| Specificity | Human ARSA |
| Immunogen | Recombinant Human ARSA protein (Catalog#10449-H08H) |
| Preparation | Produced in rabbits immunized with purified, recombinant Human ARSA (rh ARSA; Catalog#10449-H08H; P15289-1; Met 1-Ala 507) and then biotinylated. ARSA specific IgG was purified by human ARSA affinity chromatography and then biotinylated. |
| Source | Polyclonal Rabbit IgG |
| Purification | Protein A & Antigen Affinity |
| Formulation | 0.2 μm filtered solution in PBS |
| Conjugate | Biotin |
| Form | Liquid |
| Shipping | This antibody is shipped as liquid solution at ambient temperature. Upon receipt, store it immediately at the temperature recommended below. |
| Storage | This antibody can be stored at 2℃-8℃ for one month without detectable loss of activity. Antibody products are stable for twelve months from date of receipt when stored at -20℃ to -80℃. Preservative-Free. Avoid repeated freeze-thaw cycles. |
Synonyms: Anti-ASA Antibody; Anti-MLD Antibody
Background Information
| Full Name | arylsulfatase A |
| Description | Arylsulfatase A (ARSA) is synthesized as a 52KDa lysosomal enzyme. It is a member of the sulfatase family that is required for the lysosomal degradation of cerebroside-3-sulfate, a sphingolipid sulfate ester and a major constituent of the myelin sheet. Arylsulfatase A is activated by a required co- or posttranslational modification with the oxidation of cysteine to formylglycine. Metachromatic leukodystrophy (MLD) is a lysosomal storage disease in the central and peripheral nervous systems with severe and progressive neurological symptoms caused by the deficiency of Arylsulfatase A. Deficiency of this enzyme is also found in apparently healthy individuals, a condition for which the term pseudodeficiency is introduced. ARSA forms dimers after receiving three N-linked oligosaccharides in the endoplasmic reticulum, and then the dimers are transported to the Golgi where they receive mannose 6-phosphate recognition markers. And thus, ARSA is transported and delivered to dense lysosomes in a mannose 6-phosphate receptor-dependent manner. It has been shown that within the lysosomes, the ARSA dimers can oligomerize to an octamer in a pH-dependent manner. The ARSA deficiency leads to metachromatic leucodystrophy (MLD), a lysosomal storage disorder associated with severe and progressive demyelination in he central and peripheral nervous system. Additionally, the serum level of arylsulfatase A might be helpful in diagnosis of lung and central nervous system cancer. |
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